Iron inclusions in plasma cells

Abstract

Copper deficiency is a rare cause of pancytopenia. We report findings in a 26-year-old female with history of Wilson’s disease diagnosed 13 years previously who was found to be pancytopenic. She was admitted for replacement of a muscle relaxant (baclofen) pump. Full blood examination showed haemoglobin 73 g/L, MCV 90 fl, MCH 26 pg, WCC 1.5 × 109/L, neutrophils 0.7 × 109/L and platelet count 115 × 10 /L. Bone marrow examination revealed a normocellular marrow with dyserythropoeisis, sequential myeloid maturation and increased megakaryocytes. Erythroid and myeloid precursors showed vacuoles in the cytoplasm. There were prominent iron positive inclusions in the plasma cells in the iron stain. The bone marrow findings seen in this patient have been classically described in copper deficiency. One of the treatment modalities in Wilson’s disease is zinc therapy to prevent absorption of copper. The patient was on long term Zinc supplementation (220 mg three times a day). Her serum zinc level was 19.2umol/L, serum copper 0.1 μmol/L and ceruloplasmin 0.02 g/L. While zinc supplementation can be an effective treatment for Wilson’s disease, overtreatment can induce copper deficiency leading to cytopenias with characteristic bone marrow appearance. Recognition is important as copper deficiency can be associated with neurological deterioration due to peripheral neuropathy.